Uveitis and rheumatic conditions

The uvea is the pigmented middle layer of the eye, and the iris is part of it; uveitis is inflammation of that layer. Doctors name it by location. Inflammation at the front of the eye is anterior uveitis, also called iritis; just behind that is intermediate uveitis; at the back of the eye, near the retina, is posterior uveitis; and when the whole uvea is inflamed the term is panuveitis. Anterior uveitis is seen far more than the other forms, and it is the one rheumatic disease goes with most often.

It usually makes itself known as a painful, red eye that flinches from bright light, coming on over hours or a day or two. Deeper in the eye the inflammation can be quieter: vision blurs or floaters appear, with little or no pain.

The spondyloarthritis family of inflammatory conditions, many of whose members are linked to the genetic marker HLA-B27, is strongly associated with anterior uveitis. Axial spondyloarthritis, psoriatic arthritis and reactive arthritis belong to it, as does enteropathic arthritis, the arthritis that can come with inflammatory bowel disease. Typically the uveitis here is acute: it affects one eye at a time, comes on quickly, settles with treatment, and can return.

Different patterns of eye inflammation go with other rheumatic conditions. With Behçet's disease the inflammation may be at the front of the eye or at the back, and it has to be watched carefully; sarcoidosis is a recognised cause as well. Adults who had juvenile idiopathic arthritis as children may bring with them a history of chronic anterior uveitis, a kind that often gave no symptoms and came to light only through regular eye screening, and that history still matters to their care in adult life.

What matters most to a rheumatologist is that uveitis can be the first sign of a condition that has not yet been diagnosed. Mild or on-and-off back or joint symptoms may have sat in the background for some time, and it is an attack of acute anterior uveitis that first takes someone to a doctor. An ophthalmologist who sees uveitis recur, or who finds it in both eyes or alongside back, joint, skin or bowel symptoms, will often ask for the condition behind it to be looked for, which is where a rheumatology review comes in.

Where the inflammation is decides what you notice. Rheumatologists mostly meet the acute anterior form, and it tends to bring:

• Redness and pain in the eye, often one eye alone
• Light becoming hard to bear (photophobia)
• Vision that is blurred, or seems dimmer than before
• A pupil that looks small or irregular, or an ache that focusing makes worse

Deeper inflammation, in the intermediate or posterior parts of the eye, may cause only floaters or blurring, with little or no pain, and is easy to overlook as a result. The same quietness, and the fact that the chronic uveitis tied to childhood arthritis may give no symptoms at all, is why some of those with a known risk have their eyes checked at intervals even when nothing feels wrong.

Pain and redness in an eye that cannot stand light are not something to wait and see about: such an eye is seen at an eye casualty or urgent eye clinic the same day, and the urgency is repeated below.

Diagnosing the uveitis itself is the eye specialist's work. The examination uses a slit lamp, a microscope through which the inflammatory cells inside the eye can be seen, and includes a check of the eye pressure and a look at the back of the eye. It confirms that uveitis is present, shows where it sits and guides the treatment.

The rheumatologist comes in once the uveitis is confirmed, especially when it recurs, involves both eyes, or arrives with joint, back, skin or bowel symptoms, and the task is to find an underlying condition or rule one out. That may mean a careful history and examination; blood tests, the inflammatory markers and the genetic marker HLA-B27 among them; and, if spondyloarthritis is suspected, imaging of the spine or of the joints affected. Point-of-care ultrasound is part of the consultation with Dr Borukhson, and a joint that is involved can be scanned with it for inflammation (more on the scan itself is on the ultrasound clinic page). It is often the eye and the joints considered together, in a single assessment, that produce a clear diagnosis.

Ophthalmology leads the treatment, and the type and severity of the uveitis decide it. Steroid drops are the mainstay for acute anterior uveitis, and they calm the inflammation; drops that dilate the pupil are often added, because they relieve the pain and prevent the iris from sticking down. In most cases a single episode responds well, clearing in a matter of weeks; the drops are wound down gradually, not stopped abruptly.

Uveitis that keeps returning, that reaches the back of the eye or that threatens sight may call for treatment that works through the whole body, a course of steroid tablets to begin with as a rule, tapered gradually over the weeks that follow, or in some circumstances a steroid injection around or into the eye; the eye specialist leads that decision, and where a rheumatic condition lies behind the uveitis the rheumatology team shares in it. Flares can be made less frequent by conventional disease-modifying medicines, methotrexate for example, while for uveitis that is severe or keeps returning, biologic medicines are used, the anti-TNF drug adalimumab in particular, an option for active uveitis at the back of the eye that threatens sight and has not settled on steroids and an immunosuppressant; in Behçet's disease, where the eye disease can threaten sight, anti-TNF treatment is the next step after the first-line medicines, and comes in earlier where sight is at risk. Several of these medicines have the advantage of acting on the joint disease and the eye inflammation at once, so one treatment can serve both. Specialists prescribe and monitor them, and weigh benefit against risk throughout. Should you be planning a pregnancy, tell both teams: methotrexate is stopped ahead of conception, and replaced if treatment is still needed, whereas adalimumab can be continued in pregnancy where it is needed. Our guide to arthritis, autoimmune conditions and pregnancy says more about medicines before and during pregnancy.

Two specialties share uveitis, and the care works best when each knows what the other is doing. The eye team deals with the inflammation and keeps your sight safe; the rheumatology team searches for the condition that underlies it, treats it, and shares in the decisions about longer-term medicines. When the two talk to each other, a change in the eyes can prompt a look at the joints, and a change in the joints can prompt a look at the eyes.

To take a London example, the Western Eye Hospital is part of Imperial College Healthcare NHS Trust, which lets the eye and rheumatology teams work within one Trust. If an eye clinic already looks after your uveitis and you have joint, back or other symptoms still without an explanation, tell either team, so that the whole of the picture is looked at.

Because sight is at risk in uveitis, certain eye symptoms are not for a routine appointment, and a few signs elsewhere in the body cannot wait at all.

An eye that suddenly and completely loses its sight is a 999 or A&E emergency, even if it comes back after a short time. Severe illness with suspected sepsis, a high fever with shaking, confusion, rapid breathing, or feeling dramatically worse over hours, is a 999 or A&E emergency. With a fever or feeling unwell, a rash that does not fade when you press it, a stiff neck with eyes that cannot bear bright light, or skin, lips or tongue turning blue, grey, pale or blotchy is a 999 or A&E emergency too, on any treatment or none, since these can be signs of meningitis or sepsis; on black or brown skin the rash and the change of colour may be easier to see on the palms of the hands or the soles of the feet. Without the stiff neck, or without the fever or feeling unwell, an eye that light hurts is still for an eye casualty or urgent eye clinic the same day. Chest pain that is crushing, tight or like a pressure, that spreads to an arm, the jaw, the neck, the stomach or the back, that comes with breathlessness, sweating, feeling sick or feeling light-headed, or that came on suddenly and does not go away, is a 999 or A&E emergency for anyone, and so is breathlessness that is severe, with gasping, choking or no breath to get words out, as is coughing up blood. Palpitations happening now that do not stop, or that come with chest pain, breathlessness, feeling faint or fainting are a 999 or A&E emergency for anyone too, since a heart whose muscle or rhythm is affected has to be checked at once. One side of the face drooping, an arm or a leg that suddenly goes weak or numb, or speech that slurs or fails can be a stroke, as can a sudden severe headache or sudden confusion: each is a 999 or A&E emergency for anyone, in pregnancy as at any other time, and even if it stops after a short time; so is a seizure. Lips, mouth, throat or tongue that suddenly swell, a throat that feels tight, very fast breathing or a struggle to breathe or to swallow, skin, tongue or lips turning blue, grey or pale (on black or brown skin this may be easier to see on the palms of the hands or the soles of the feet), becoming suddenly very confused, drowsy or dizzy, or fainting and not being able to be woken is a 999 or A&E emergency, since this can be a severe allergic reaction, anaphylaxis, which can follow a dose of any medicine. A rash that spreads across the body, skin that blisters or peels, or lips, mouth or eyes that turn sore or swollen is a 999 or A&E emergency on adalimumab or any other medicine, since this can be a severe reaction to a medicine, such as Stevens-Johnson syndrome, which needs treating in hospital straight away, though a red, painful eye on its own, of the kind uveitis itself brings, is not what is meant. For any of these, call 999 and do not drive yourself to A&E.

Go to an eye casualty or urgent eye clinic for assessment the same day if any of these appears:

• A painful, red eye that is new, or an eye that light has begun to hurt
• Sight that has blurred, dimmed or got worse, or new floaters or flashes
• Uveitis in the past, and symptoms that feel like it coming back

Anyone with a diagnosed rheumatic condition should treat a red or painful eye as anything but minor, and have it seen at an eye casualty or urgent eye clinic the same day: vision is protected by early treatment.

If you take methotrexate, adalimumab or another medicine that damps down the immune system, for the uveitis or for the condition behind it, a fever or feeling generally unwell needs an assessment that is urgent, on the same day, from your GP or NHS 111, or from the team that prescribes the medicine, whether that is the eye clinic or rheumatology, since an infection has to be treated early, and on methotrexate the same goes for a persistent cough, chest pain or breathlessness that is new or getting worse, since the lungs can become inflamed; in pregnancy, breathlessness that comes on suddenly, or when you lie down, or with dizziness, is for your midwife or maternity unit straight away. On methotrexate, adalimumab or any other medicine that damps down the immune system, being near someone with measles, chickenpox or shingles needs advice the same day too, and NHS 111 can tell you what to do, since measles and chickenpox can be dangerous when the immune system is weakened and you can catch chickenpox from someone with shingles if you have never had chickenpox. A joint that is hot and swollen, with a fever or feeling unwell, needs assessment the same day, either at A&E or by calling NHS 111, since an infected joint has to be ruled out at once; one that has become hot and very swollen suddenly, or is worsening quickly, even without a fever, needs an answer the same day too, from an urgent GP appointment, NHS 111 or a specialist clinic that can see you that day; and a joint that will take no weight at all, or that cannot be moved, is a 999 or A&E emergency, whether or not there is a fever.

In pregnancy, any bleeding, stomach pain that is severe or does not go away after a rest, reduced baby movements, a severe headache or visual disturbance, sudden swelling of the face, hands, legs or feet, or breathlessness that comes on suddenly, or when you lie down, or with dizziness, means contacting your midwife or maternity unit straight away, whatever the time, or, before a first midwife appointment, your GP surgery or early pregnancy unit, or NHS 111 when they are closed; bleeding that is heavy, or that comes with severe pain in the tummy, pain in the shoulder, or feeling sick, faint or dizzy, or passing out, is a 999 or A&E emergency, and for that call 999 and do not drive yourself to A&E; on steroid tablets, pain in the abdomen or back that comes on suddenly and severely is a 999 or A&E emergency, as the paragraph on steroids below says; a change in your sight still goes to the eye casualty as well, since in pregnancy it can have more than one cause. A high temperature in pregnancy, whatever medicines you are taking, needs checking the same day too, since a fever can be a sign of an infection, and your midwife or maternity unit, or your GP or NHS 111, can advise. In pregnancy, contact with someone who has an illness with a rash, measles, chickenpox and shingles among them, whatever medicines you are taking, means ringing your midwife or maternity unit, or your GP, straight away, since some of these infections can harm an unborn baby.

If you take steroid tablets, for the uveitis or for the condition behind it, nine more signs belong here. Serious harm you have already done to yourself, such as an overdose, or a feeling that you may be about to harm yourself is a 999 or A&E emergency, since you need help to keep you safe now, as is vomiting blood, or bleeding from the back passage that is heavy or will not stop, most of all with faintness, confusion or pain in the abdomen, or vomit that is black or dark brown, since a stomach ulcer on prednisolone can bleed, and so is sudden severe pain in the abdomen or back, since the pancreas can react to prednisolone. Vomiting that will not let your tablets stay down, or, during an illness or after an injury, becoming very drowsy or confused, or fainting, after more than a few weeks on steroid tablets, or repeated courses or injections in the past year, is a 999 or A&E emergency too, since your body may be running short of cortisol and the steroid may need to be given by injection; have your NHS Steroid Emergency Card ready. Call 999 and do not drive yourself to A&E. Thoughts of ending your life or of hurting yourself, or harm you have done to yourself that is not serious, short of the serious harm this paragraph's opening sentences put at 999, which steroids can bring on: ask your GP to see you that day or call NHS 111, and Samaritans answer at any hour on 116 123. The same-day route above, from your GP, NHS 111 or the team that prescribes the medicine, is the answer to a marked change in mood or behaviour, confusion, or seeing or hearing things that are not there, since prednisolone can do this, the more so the higher the dose, though confusion that comes on suddenly is a 999 or A&E emergency, as the sentence on the signs of a stroke above says, and so, after more than a few weeks on steroid tablets, or repeated courses or injections in the past year, is becoming very drowsy or confused during an illness or after an injury, as this paragraph's opening sentences say; to unusual thirst or hunger, passing urine more often, or feeling sleepy or confused, swelling of the arms or legs, bruising or bleeding that is not normal, black or dark red stools, severe stomach pain, severe back pain, a severe upset stomach, being sick, or very bad dizziness or passing out, since each can be a steroid effect that needs checking without delay, though pain in the abdomen or back that comes on suddenly and severely is a 999 or A&E emergency, as this paragraph's opening sentences say, and so, after more than a few weeks on steroid tablets, or repeated courses or injections in the past year, is vomiting that will not let your tablets stay down, or, during an illness or after an injury, becoming very drowsy or confused, or fainting, while in pregnancy, bleeding that is not normal, and stomach pain that is severe, belong to the paragraph on pregnancy above; to new chest pain, new palpitations, or breathlessness that is new or getting worse, since a steroid can unsettle the heartbeat and bring on breathlessness, and both are signs to have the heart and lungs checked, though palpitations happening now that do not stop, or that come with chest pain, breathlessness, feeling faint or fainting, are a 999 or A&E emergency, as the sentence on palpitations above says, and in pregnancy, breathlessness that comes on suddenly, or when you lie down, or with dizziness, is for your midwife or maternity unit straight away; and to contact with measles, chickenpox or shingles, since these infections can be severe on steroids and a medicine to protect you may be needed.

Long-standing, stable joint symptoms can wait for routine review. If you take methotrexate, another DMARD, a steroid or a biologic, the warning signs that belong to each are set out in our guides to methotrexate, DMARDs, steroids and biologics and JAK inhibitors. If you cannot tell how urgent something is, asking is never a mistake.

Uveitis matters for its own sake, and it can also point to something else. Sometimes it is a rheumatic condition making its first appearance, one that might otherwise have gone unrecognised for longer; where the diagnosis already exists, an attack is a reason for the two teams to look at the whole picture together. A specialist rheumatology assessment, working alongside your eye team, can search for a cause, read it against your other symptoms, and put together a plan that keeps both joints and sight safe. Once the eye is looked at with the rest of the body in view, a frightening episode often becomes a clear diagnosis with a plan to match.

Is there always a rheumatic condition behind uveitis?

No. A one-off episode, with nothing behind it, is a common experience; in some people an infection is the cause. Even so, the anterior form, at the front of the eye, has a strong link with the spondyloarthritis family, axial spondyloarthritis and psoriatic arthritis among them, and an episode can be the first sign that one of these conditions is present. Uveitis that recurs, that affects both eyes, or that comes with back, joint, skin or bowel symptoms therefore prompts a search for an underlying inflammatory condition, and a rheumatology review is where that search is made.

How quickly does a red, painful eye need seeing?

If an eye suddenly loses its sight completely, even for a short time, that is a 999 or A&E emergency, and so is an eye that light hurts with a stiff neck and a fever or feeling unwell, since that can be meningitis. Call 999 and do not drive yourself to A&E. The same day: an eye casualty or urgent eye clinic should see a newly painful or red eye, an eye that light hurts, or vision that has blurred, or new floaters or flashes, that day, because untreated uveitis can threaten sight; in pregnancy, any change in sight, blurring or flashing lights among them, is also for your midwife or maternity unit straight away, since it can be a sign of pre-eclampsia. And if uveitis is something you have had before, symptoms that feel like it coming back should be seen at an eye casualty or urgent eye clinic the same day too.

Ankylosing spondylitis, its relatives, and uveitis: what is the link?

A shared genetic marker, HLA-B27, runs through many of the spondyloarthritis conditions: axial spondyloarthritis (ankylosing spondylitis is part of it), reactive arthritis, and the arthritis that goes with inflammatory bowel disease. Carrying the marker raises the chance of acute anterior uveitis, which typically starts quickly in one eye at a time, settles with treatment and can come back.

What is the treatment for uveitis?

An eye specialist leads it. Steroid eye drops are the usual answer to acute anterior uveitis, and drops that widen the pupil are frequently added for the pain; in most cases a single episode has cleared within a few weeks. If the uveitis keeps recurring or puts vision at risk, the eye specialist may bring in treatment that works through the whole body, a course of steroid tablets first as a rule and then medicines such as methotrexate or adalimumab, with the rheumatology team sharing that decision where a rheumatic condition lies behind the uveitis, and several of these treat the joint disease and the eye inflammation together.

I have had uveitis. Why am I being sent to a rheumatologist?

Because a rheumatic condition can announce itself through uveitis, particularly a second attack, uveitis in both eyes, or symptoms elsewhere, in the back, the joints, the skin or the bowel. Examination, blood tests and, when a joint is involved, ultrasound in the clinic let a rheumatology assessment find, or rule out, a condition such as axial spondyloarthritis or Behçet's disease. A cause that is found can be treated, and that treatment can mean fewer flares in the eye as well.